Author + information
- Division of Cardiology, Corrigan Minehan Heart Center, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts
- ↵∗Address for correspondence:
Dr. Ami B. Bhatt, Massachusetts General Hospital, 55 Fruit Street, Yawkey 5B, Boston, Massachusetts 02114.
As pediatric congenital heart management continues to improve, the number of women who have congenital heart disease (CHD) and are of childbearing age is increasing. In this issue of the Journal, Schlichting et al. (1) have added to the published reports a robust assessment of over 22 million deliveries in the United States between 2008 and 2013, nearly 18,000 of which were deliveries by women with CHD. Despite the caveats of using administrative data, the authors have delineated the increased odds of maternal comorbidities, higher rates of adverse events, longer lengths of stay, and higher total charges in the population of pregnant women with CHD, when compared with those without CHD. There are several key lessons from this research: maternal comorbidities are often predictable and, therefore, manageable—if not preventable—and their presence does not necessitate obstetric intervention. Nonetheless, obstetric care is an important mechanism for identifying individuals who have fallen out of cardiac care. Additionally, individuals with severe CHD require longitudinal planning that begins far before pregnancy is considered.
Comorbidities: Predictable and Often Preventable
In this study, 17,729 delivery hospitalizations were in women with CHDs (which translates into 77.5 deliveries per 100,000). Mild-to-moderate CHDs were present in 70% (n = 12,366) of CHD patients; 25% of patients possessed an isolated atrial septal defect (ASD) (n = 4,375); and the remaining 5% of patients had severe CHDs (n = 988). Women with CHDs had greater rates of comorbidities that may complicate delivery, including cardiomyopathy, valvular heart disease, pulmonary hypertension, chronic systemic hypertension, cardiac conduction disorder, anemia, and nongestational diabetes, with the prevalence of these comorbidities continuing to rise in the CHD population. This highlights an at-risk population in whom these pre-conception diagnoses should prompt increased vigilance, frequent prenatal evaluation, and clear documentation of labor and delivery contingency planning. Multidisciplinary care involving cardiology, obstetrics, and obstetric anesthesia for individuals with CHD who become pregnant is essential, even in the mild-to-moderate CHD population.
Presence of maternal comorbidities does not equate with obstetric intervention
The investigators report that assisted delivery was used to a greater degree for women with CHD of any severity. Considering the low incidence of severe maternal CHD in this study, the mild-to-moderate disease population significantly contributed to the elevated adjusted odds ratios for various methods of delivery assistance, including cesarean section, induction, and operative vaginal delivery.
Future research and registries will better define factors leading to greater odds for operative vaginal delivery and cesarean section in pregnant women with mild-to-moderate CHDs. Although clinical concerns for the mother or fetus, such as maternal hemodynamic instability, might drive the mechanism of delivery, it is likely that systems issues, such as the lack of a delivery plan, inadequate understanding of CHD, or the absence of multidisciplinary cardio-obstetric care, influence the decision to choose an assisted delivery. This study’s low rates of fetal distress suggest that fetal indications did not drive assisted obstetric intervention. Improved expectant maternal cardiovascular clinical management, along with strategic education, planning, and communication, may lead to decreased rates of obstetric intervention in the future. Fortunately, while awaiting registry data, the personalized assessment of labor and delivery risk/benefit and contingency planning can begin now as a quality improvement initiative in any institution, especially those with a cardiovascular center and a maternal fetal medicine program. This kind of active, individualized planning is a mainstream expectation for all women with known or newly diagnosed cardiovascular disease during gestation. The goal is not necessarily to decrease the use of assisted delivery, but to improve the appropriate use of assisted delivery. This in turn may decrease length of stay by both allowing fewer delivery interventions in low-risk patients, and by also appropriately timing assisted delivery in higher-risk patients.
Severe CHD demands education at a younger age
In this study, only 5% of the deliveries in the CHD population occurred in women with severe disease (n = 988). The investigators demonstrate in Figure 1 that the percentage of women with severe CHDs and concomitant diagnoses of congestive heart failure, conduction and rhythm disorders, or birth defects was greater than the percentage of women with mild-to-moderate CHDs or isolated ASD with these comorbidities. Importantly, this severe population also had longer lengths of stay and higher total charges. Despite improvements in care, as we support patients with the most severe CHDs into childbearing age (2), these individuals will continue to present the highest risk for comorbidities, adverse events, and increased lengths of stay and total charges. Therefore, education on these risks and how to mitigate them, from an early age, is of great importance in the care of these patients, especially when noting the concerning finding that women possessing severe defects delivered at a younger age than those with less severe forms of CHD: Nearly 40% of individuals in this study with severe CHD were between the ages of 15 and 24 years. Importantly, the Nationwide Inpatient Sample can only identify patients who arrived at a hospital for delivery, and thus does not reflect those who became pregnant but never completed the pregnancy or did not deliver in a hospital (3).
To address young, severe-CHD patients becoming pregnant and having complications, multidisciplinary care with family planning discussions must begin at puberty. Awareness of the potential for pregnancy and age- and developmentally appropriate discussions regarding the constantly changing risk/benefit profile of pregnancy, labor, and delivery in an individual with severe CHD must be a foundation of CHD care. Contraception must be as natural a discussion as the routine queries about palpitations and shortness of breath. Recognizing the high rates of anxiety, depression, and social isolation in young individuals with chronic disease, these discussions must be undertaken within a construct of empowering the youth with severe CHD. An individual’s future family goals should be a comfortable ongoing discussion that includes development of gender identity, positive self and body image, consideration of relationships, and family planning. The ideal outcome in persons who then choose to have biological children would be to have the pregnancy and delivery guided by multidisciplinary care.
Coding caveat: Isolated ASD
The administrative grouping of small ASDs and patent foramen ovale (PFO) with larger, clinically significant ASDs results in challenges of interpretation. The authors acknowledge that the higher risk of thromboembolism identified in the isolated ASD population may be related to coding. Individuals with thromboembolic events are often investigated for ASD/patent foramen ovale and an incidental, not necessarily attributable, septal defect may be found and coded. Consequently, readers should not interpret that isolated ASD with pregnancy has a causal relationship with thromboembolism on the basis of this study.
Longitudinal, personalized, multidisciplinary care is essential to improving pregnancy outcomes in CHD. Schlichting et al. (1) have highlighted several actionable areas of cardio-obstetric care that may bend the curve of care within the next few years. Although administrative data might not shed light on chronic disease progression and risk prediction, this analysis offers clear direction for process improvement in the management of the individuals with CHD of childbearing age. Pre-conception counseling must begin earlier than we think, especially in severe CHD, because teenage pregnancy is a significant problem. Because loss to follow-up is relatively common in adult CHD, obstetric care is an important reintroduction to longitudinal cardiac care and should be facilitated in the pre-conception or early stages of pregnancy, as soon as a patient presents. For patients with mild-to-moderate CHD, multidisciplinary care can alert the obstetric team to potential comorbidities and adverse events (4). The hemodynamic changes in pregnancy are well defined, and several risk assessment tools exist to predict maternal cardiovascular events. This allows a multidisciplinary team to plan for appropriate, active surveillance during pregnancy, labor, delivery, and postpartum, improving resource utilization and lessening both patient and care team anxiety. Community-based care is possible in CHD, particularly for women with simple CHDs who generally tolerate pregnancy well. One-time CHD consultation is always encouraged to ensure the anatomic defects, repairs, and ensuing physiological changes are well-defined. Women with unrepaired disease, complex congenital defects, or significant residual defects should be managed in centers with a multidisciplinary team that consists of adult CHD and high-risk maternal fetal providers, and that includes well-orchestrated pre-conception counseling, pregnancy, labor, and delivery planning, fetal evaluation, and post-partum management and surveillance. With adequate planning, the future is bright for pregnancy and CHD.
Wish you an easy delivery? Sure, but chance favors one that includes a longitudinal multidisciplinary team.
↵∗ Editorials published in the Journal of the American College of Cardiology reflect the views of the authors and do not necessarily represent the views of JACC or the American College of Cardiology.
Dr. Bhatt has reported that she has no relationships relevant to the contents of this paper to disclose.
- 2019 American College of Cardiology Foundation
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